Other necrotizing vasculopathies (M31)

The ICD-10 code M31 series is used to classify various specific types of necrotizing vasculopathies, which involve inflammation and destruction of blood vessel walls. These codes apply to diseases such as hypersensitivity angiitis, thrombotic microangiopathy, Wegener's granulomatosis, and giant cell arteritis, among others.

This group covers rare but significant conditions characterized by vessel wall damage and immune complex involvement. For example, code M31.0 refers to hypersensitivity angiitis; also called pulmonary renal syndrome or Goodpasture's syndrome; which involves immune reactions causing vessel inflammation. The codes M31.1 through M31.19 identify various forms of thrombotic microangiopathy, including those linked to hematopoietic stem cell transplantation. Codes M31.3 and its subtypes specify Wegener's granulomatosis (now known as granulomatosis with polyangiitis), a multisystem vasculitis often affecting kidneys and respiratory tract. The section also includes M31.4 for Takayasu's disease and M31.5 and M31.6 for different forms of giant cell arteritis. Medical coders can rely on these ICD-10 codes for precise documentation of these immune-mediated, necrotizing vascular diseases.

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Giant Cell Arteritis

A systemic autoimmune disorder that typically affects medium and large ARTERIES, usually leading to occlusive granulomatous vasculitis with transmural infiltrate containing multinucleated GIANT CELLS. The TEMPORAL ARTERY is commonly involved. This disorder appears primarily in people over the age of 50. Symptoms include FEVER; FATIGUE; HEADACHE; visual impairment; pain in the jaw and tongue; and aggravation of pain by cold temperatures. (From Adams et al., Principles of Neurology, 6th ed)

Granulomatosis with Polyangiitis

A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and KIDNEYS. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against MYELOBLASTIN.

Microscopic Polyangiitis

A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.

Polymyalgia Rheumatica

A syndrome in the elderly characterized by proximal joint and muscle pain, high erythrocyte sedimentation rate, and a self-limiting course. Pain is usually accompanied by evidence of an inflammatory reaction. Women are affected twice as commonly as men and Caucasians more frequently than other groups. The condition is frequently associated with GIANT CELL ARTERITIS and some theories pose the possibility that the two diseases arise from a single etiology or even that they are the same entity.

Rheumatoid Vasculitis

Necrotizing VASCULITIS of small and medium size vessels, developing as a complication in RHEUMATOID ARTHRITIS patients. It is characterized by peripheral vascular lesions, cutaneous ULCERS, peripheral GANGRENE, and MONONEURITIS MULTIPLEX.